综合性手术对Kasabach-Merritt现象的疗效分析  

Analysis of efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon

作　　者：杨光 龚毓宾 孙正伟 符琼扬 宋畅 董长宪 Yang Guang;Gong Yubin;Sun Zhengwei;Fu Qiongyang;Song Chang;Dong Changxian(Department of Hemangioma,People′s Hospital of Zhengzhou University,Henan Provincial People′s Hospital,Zhengzhou 450003,China)

机构地区：[1]郑州大学人民医院河南省人民医院血管瘤科,450003

出　　处：《中华皮肤科杂志》2020年第7期557-560,共4页Chinese Journal of Dermatology

摘　　要：目的分析综合性外科手术治疗Kasabach-Merritt现象(KMP)的疗效。方法回顾性分析2017年11月至2019年3月在河南省人民医院血管瘤科接受外科治疗的18例KMP患者的临床资料。术前使用糖皮质激素及免疫球蛋白提升患者血小板计数,对糖皮质激素不敏感者术前12~24 h予单次输注大剂量血小板,血小板<30×109/L者按0.3个治疗量/kg输注,血小板≥30×109/L者按0.2个治疗量/kg输注,总量不超过1个治疗量(1个治疗量总液体量为200~250 ml,约含血小板2.5×1011个)。术中尽可能剥离肿瘤组织,对大面积肿瘤术中缝合困难者,使用自体原位植皮术,对术后有功能障碍可能性的患儿请康复师会诊,予个体化功能锻炼,并促进功能恢复。结果纳入18例患者,男9例,女9例,平均年龄73 d(范围7~354 d)。皮损位于四肢3例,躯干11例,头面颈部4例。手术成功17例,1例死亡。手术成功的患儿中,16例血小板及凝血功能于术后1周内均恢复至正常,1例患儿术后经规律口服西罗莫司后血小板及凝血功能也恢复正常;术后患儿均能保持良好运动能力。结论综合性外科手术治疗KMP疗效肯定,起效迅速,不良反应少。Objective To analyze efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon(KMP).Methods Clinical data were collected from 18 patients with KMP who underwent surgical treatment in Department of Hemangioma,Henan Provincial People′s Hospital from November 2017 to March 2019,and analyzed retrospectively.Preoperative treatment included preoperative use of glucocorticoids and immunoglobulins to increase platelet counts in patients.For patients irresponsive to glucocorticoids,a single large-dose of platelets was infused 12-24 hours before surgery.The platelet dose for infusion should be 0.3 and 0.2 therapeutic dose(TD)/kg respectively for patients with platelet counts lower than 30×109/L and those with platelet counts higher than 30×109/L,and the total infusion dose should be no more than 1 TD(containing about 2.5×1011 platelets in 200-250 ml of infusion solution).During the operation,tumor tissues were removed as much as possible;if there were difficulties in suturing in patients with large tumors,in situ autologous skin grafting would be performed;for children with potential postoperative functional dysfunction,a clinical consultation with rehabilitation specialists was given,and individualized functional exercises were prescribed to promote functional restoration.Results Eighteen patients were enrolled,including 9 males and 9 females,with an average age of 73 days(range,7-354 days).Skin lesions were located on the extremities in 3 cases,on the trunk in 11,and on the head,face and neck in 4.Surgeries were successful in 17 patients,but 1 died.Among the 17 patients with successful operation,platelet counts and coagulation function returned to normal within 1 week after the surgery in 16,and did not returned to normal until after regular oral administration of sirolimus in 1.Favorable movement ability was maintained in all the children.Conclusion Comprehensive surgical treatment shows rapid efficacy with less adverse reactions in the therapy of KMP.

关 键 词：KASABACH-MERRITT综合征 治疗结果 手术疗法 

分 类 号：R726.5[医药卫生—儿科]